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Inherited deficiency of the initiator molecules of the lectin-complement pathway
The complement system is an important immune system.
Its activation results in membranolytic elimination of
microbes and opsonization. The classical, alternative and
lectin pathways (LP) activate complement. Either mannan-
binding lectin (MBL), ficolin-1, ficolin-2 or ficolin-3 initiate
the LP through associated serine protease (MASP-2)
after binding to microorganisms’surface carbohydrate
patterns. Genetic polymorphisms behind MBL deficiency
Bjarnadottir H, Ludviksson BR
are rather common. Numerous studies indicate that
MBL deficiency is a risk factor for invasive and recurrent
infections, especially when other immune systems are
immature, deficient or compromised. Research in ficolins
is limited but last year ficolin-3 deficiency was described.
This review focuses on these recently WHO defined
immunodeficiencies.
Inherited deficiency of the initiator molecules of the lectin-complement pathway. /ce/ Med J 2010; 96; 611-7
Key words: innate immunity, mannan-binding lectin (MBL), ficolins, lectin pathway (LP), complement activation.
Correspondance: Björn Rúnar Lúðviksson, bjornlud@landspitali.is
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Barst: 26. október 2009, - samþykkt til birtingar: 9. september 2010
Hagsmunatengsl: Engin
LÆKNAblaðið 2010/96 61